English

Mitochondrial Calcium Signalling

Data update as of 31/12/2025

44-FOTO GRUPO SEÑALIZACIÓN MIT CA2025 DATA

Leaders

Araceli Del Arco Martínez
adelarco@cbm.uam.es
Consult CV (225.7 KB ) (4 pages)
Beatriz Pardo Merino
bpardo@cbm.csic.es
Consult CV (321.5 KB ) (5 pages)
Jorgina Satrústegui Gil-Delgado
jsatrustegui@cbm.csic.es
Consult CV (389.6 KB ) (5 pages)

Research

Basic

Location

Laboratory 321

Severo Ochoa Molecular Biology Centre (CBMSO)

Autonomous University of Madrid (UAM).

C/ Nicolás Cabrera nº 1, Cantoblanco, 28049 Madrid (Spain).

Keywords

Calcium signalling, mitochondrial physiology and pathophysiology, neuronal metabolism, hepatic metabolism, and diseases associated with mitochondrial transporters.

Gender perspective

Calcio-01

Summary of activities

The research conducted by our group has focused on mitochondrial pathophysiology in the brain and liver, particularly in relation to the activity of mitochondrial aspartate/glutamate carriers (AGCs). In this context, we have demonstrated that, in hepatocytes deficient in SLC25A13/Citrin, the liver-specific isoform responsible for Citrullinemia Type II (CTLN2), exogenous expression of SLC25A12/Aralar, the isoform predominantly expressed in excitable tissues, is functional and partially reverses the pathological phenotype. This finding opens new therapeutic avenues for the treatment of liver diseases associated with SLC25A13/Citrin deficiency, providing a basis for the development of innovative strategies aimed at restoring mitochondrial function and improving disease outcomes.

Contributions to society

Citrullinemia Type II (CTLN2) and neonatal intrahepatic cholestasis caused by Citrin deficiency, both resulting from SLC25A13/Citrin deficiency, are rare disorders that are particularly prevalent in certain regions of Asia. Effective treatments for these conditions remain limited, highlighting the need to identify and develop novel therapeutic strategies. The group's findings provide new insights into the pathophysiology of these diseases and open promising avenues for the development of innovative therapies aimed at restoring mitochondrial function and improving clinical outcomes for affected patients.

Collaborations

National Collaborations: J.M. Cuezva (Severo Ochoa Molecular Biology Centre [CBMSO], Autonomous University of Madrid); María José Casarejos (Ramón y Cajal Health Research Institute [IRYCIS]); Gloria González-Aseguinolaza (Centre for Applied Medical Research [CIMA], Pamplona); Alberto Paradela (National Biotechnology Centre [CNB-CSIC]); and María Luz Martínez-Chantar (CIC bioGUNE, Bilbao).

International Collaborations: E.R.S. Kunji (Medical Research Council, University of Cambridge, United Kingdom); and T. Saheki (Kagoshima University, Japan).